Child's Nervous System

, Volume 34, Issue 8, pp 1489–1495 | Cite as

Distinct demographic profile and molecular markers of primary CNS tumor in 1873 adolescent and young adult patient population

  • Rohit Vadgaonkar
  • Sridhar Epari
  • Girish Chinnaswamy
  • Rahul Krishnatry
  • Raees Tonse
  • Tejpal Gupta
  • Rakesh JalaliEmail author
Original Paper



We present detailed demographic profile, tumor types, and their molecular markers in adolescent and young adult (AYA) patients of age group between 15 and 39 years with primary central nervous system (PCNS) tumors, and compare with pediatric and adult patient populations.


Demographic- and disease-related information of 1873 PCNS tumor patients of age 15–39 years registered between 1 January 2011 and 31 December 2015 at our institution was analyzed with respect to their demographics and tumor subtypes. Various molecular markers for glial tumors and subgroup classification of medulloblastoma were evaluated for AYA, pediatric, and older adult patient populations.


AYA constituted 27% of all PCNS. Median age was 29 years. Glial tumors (62.36%) comprised the largest tumor type with astrocytoma (38.55%) being the most common histology. Glioblastoma (51.52%) was the commonest astrocytic tumor with 74.67% of them being isocitrate dehydrogenase 1 (IDH1) negative and 41.38% with O (6)-methylguanine DNA methyltransferase (MGMT) promoter methylated. Diffuse astrocytoma and glioblastoma showed significantly higher IDH1 positivity and loss of alpha-thalassemia/mental retardation syndrome X-linked (ATRX) for AYAs as compared to pediatric and adult patient populations (p < 0.0001). Medulloblastoma (73.8%) was the most commonly diagnosed embryonal tumor, with sonic hedgehog (SHH) being the commonest molecular subtype (48%). Younger patients among AYA population presents with pediatric type of tumor spectrum, while older ones present with more aggressive tumor subtypes.


This is among the few studies reporting spectrum of PCNS tumor in AYA population with distinct tumor subtypes and molecular profiles. AYA patient populations may need special attention with appropriately designed clinical trials.


Gliomas Medulloblastoma Adolescent and young adults Teenage and young adults 



We thank all our members of our neuro-oncology group as well as neurosurgery colleagues from other hospitals for their patient referrals and management. Special thanks to Ms. Nazia Bano and Ms. Amita Wadwekar for their help in maintaining databases.

Funding information

We are grateful to the Brain Tumour Foundation of India for providing us the financial assistance for conducting the molecular marker research component of the study.

Compliance with ethical standards

Conflict of interest

None of the authors have any conflict of interest to declare or any financial disclosure.

Supplementary material

381_2018_3785_MOESM1_ESM.docx (15 kb)
ESM 1 (DOCX 15 kb)


  1. 1.
    Bleyer A, Ferrari A, Whelan J, Barr RD (2017) Global assessment of cancer incidence and survival in adolescents and young adults. Pediatr Blood Cancer 64(9):e26497CrossRefGoogle Scholar
  2. 2.
    Ostrom QT, Gittleman H, de Blank PM, Finlay JL, Gurney JG, McKean-Cowdin R, Stearns DS, Wolff JE, Liu M, Wolinsky Y, Kruchko C, Barnholtz-Sloan JS (2016) American brain tumor association adolescent and young adult primary brain and central nervous system tumors diagnosed in the United States in 2008–2012. Neuro-Oncology 18(Suppl 1):i1–i50CrossRefPubMedGoogle Scholar
  3. 3.
    Kunder R et al (2013) Real-time PCR assay based on the differential expression of microRNAs and protein-coding genes for molecular classification of formalin-fixed paraffin embedded medulloblastomas. Neuro Oncol 15(12):1644–1651CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Husson O, Zebrack B, Block R, Embry L, Aguilar C, Hayes-Lattin B, Cole S (2017) Personality traits and health-related quality of life among adolescent and young adult cancer patients: the role of psychological distress. J Adolesc Young Adult Oncol 6:358–362. CrossRefPubMedGoogle Scholar
  5. 5.
    Keegan THM, Ries LAG, Barr RD, Geiger AM, Dahlke DV, Pollock BH, Bleyer WA, for the National Cancer Institute Next Steps for Adolescent and Young Adult Oncology Epidemiology Working Group (2016) Comparison of cancer survival trends in the United States of adolescents and young adults with those in children and older adults. Cancer 122(7):1009–1016CrossRefPubMedGoogle Scholar
  6. 6.
    Bleyer A, Budd T, Montello M (2006) Adolescents and young adults with cancer: the scope of the problem and criticality of clinical trials. Cancer 107(7 Suppl):1645–1655CrossRefPubMedGoogle Scholar
  7. 7.
    Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P (2007) The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 114(2):97–109CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Fritz A, Percy C, Jack A, Shanmugaratnam K, Sobin L, Parkin DM (2000) International classification of diseases for oncology. World Health Organization, GenevaGoogle Scholar
  9. 9.
    Jalali R, Datta D (2008) Prospective analysis of incidence of central nervous tumors presenting in a tertiary cancer hospital from India. J Neuro-Oncol 87(1):111–114CrossRefGoogle Scholar
  10. 10.
    Jaiswal J, Shastry AH, Ramesh A, Chickabasaviah YT, Arimappamagan A, Santosh V (2016) Spectrum of primary intracranial tumors at a tertiary care neurological institute: a hospital-based brain tumor registry. Neurol India 64(3):494–501CrossRefPubMedGoogle Scholar
  11. 11.
    Huse JT, Wallace M, Aldape KD, Berger MS, Bettegowda C, Brat DJ, Cahill DP, Cloughesy T, Haas-Kogan DA, Marra M, Miller CR, Nelson SJ, Salama SR, Soffietti R, Wen PY, Yip S, Yen K, Costello JF, Chang S (2014) Where are we now? And where are we going? A report from the Accelerate Brain Cancer Cure (ABC2) low-grade glioma research workshop. Neuro-Oncology 16(2):173–178CrossRefPubMedGoogle Scholar
  12. 12.
    Balss J, Meyer J, Mueller W, Korshunov A, Hartmann C, von Deimling A (2008) Analysis of the IDH1 codon 132 mutation in brain tumors. Acta Neuropathol 116(6):597–602CrossRefPubMedGoogle Scholar
  13. 13.
    Vigneswaran K, Neill S, Hadjipanayis CG (2015) Beyond the World Health Organization grading of infiltrating gliomas: advances in the molecular genetics of glioma classification. Ann Transl Med 3(7):95PubMedPubMedCentralGoogle Scholar
  14. 14.
    Hegi ME, Diserens AC, Gorlia T, Hamou MF, de Tribolet N, Weller M, Kros JM, Hainfellner JA, Mason W, Mariani L, Bromberg JEC, Hau P, Mirimanoff RO, Cairncross JG, Janzer RC, Stupp R (2005) MGMT gene silencing and benefit from temozolomide in glioblastoma. N Engl J Med 352(10):997–1003CrossRefPubMedGoogle Scholar
  15. 15.
    Wiestler B, Capper D, Holland-Letz T, Korshunov A, von Deimling A, Pfister SM, Platten M, Weller M, Wick W (2013) ATRX loss refines the classification of anaplastic gliomas and identifies a subgroup of IDH mutant astrocytic tumors with better prognosis. Acta Neuropathol 126(3):443–451CrossRefPubMedGoogle Scholar
  16. 16.
    Kool M, Korshunov A, Remke M, Jones DTW, Schlanstein M, Northcott PA, Cho YJ, Koster J, Schouten-van Meeteren A, van Vuurden D, Clifford SC, Pietsch T, von Bueren AO, Rutkowski S, McCabe M, Collins VP, Bäcklund ML, Haberler C, Bourdeaut F, Delattre O, Doz F, Ellison DW, Gilbertson RJ, Pomeroy SL, Taylor MD, Lichter P, Pfister SM (2012) Molecular subgroups of medulloblastoma: an international meta-analysis of transcriptome, genetic aberrations, and clinical data of WNT, SHH, group 3, and group 4 medulloblastomas. Acta Neuropathol 123(4):473–484CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Rohit Vadgaonkar
    • 1
  • Sridhar Epari
    • 1
  • Girish Chinnaswamy
    • 1
  • Rahul Krishnatry
    • 1
  • Raees Tonse
    • 1
  • Tejpal Gupta
    • 1
  • Rakesh Jalali
    • 1
    Email author
  1. 1.Neuro-Oncology Disease Management GroupTata Memorial CentreMumbaiIndia

Personalised recommendations